Adjunctive therapies in idiopathic pulmonary fibrosis—where do we stand?
IPF is a restrictive lung disease that is characterized by dyspnea, non-productive cough and progressive loss of lung function. Some patients with IPF will have a slow and steady decline in their lung function, while others can experience a more rapid deterioration in their health and documented lung functions (1). The pathogenesis of IPF [histologically referred to as usual interstitial pneumonia (UIP)], is incompletely understood. However, it may involve a number of genes including surfactant protein C and telomerase mutations, resultant pneumocyte senescence, and aberrant repair pathways hallmarked by progressive pulmonary fibrosis (2,3).